Display options
Share it on

Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-48. doi: 10.1164/rccm.201308-1483ST.

An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias.

American journal of respiratory and critical care medicine

William D Travis, Ulrich Costabel, David M Hansell, Talmadge E King, David A Lynch, Andrew G Nicholson, Christopher J Ryerson, Jay H Ryu, Moisés Selman, Athol U Wells, Jurgen Behr, Demosthenes Bouros, Kevin K Brown, Thomas V Colby, Harold R Collard, Carlos Robalo Cordeiro, Vincent Cottin, Bruno Crestani, Marjolein Drent, Rosalind F Dudden, Jim Egan, Kevin Flaherty, Cory Hogaboam, Yoshikazu Inoue, Takeshi Johkoh, Dong Soon Kim, Masanori Kitaichi, James Loyd, Fernando J Martinez, Jeffrey Myers, Shandra Protzko, Ganesh Raghu, Luca Richeldi, Nicola Sverzellati, Jeffrey Swigris, Dominique Valeyre,

PMID: 24032382 PMCID: PMC5803655 DOI: 10.1164/rccm.201308-1483ST

Abstract

BACKGROUND: In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical "gold standard" of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs.

PURPOSE: The objective of this statement is to update the 2002 ATS/ERS classification of IIPs.

METHODS: An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011.

RESULTS: Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis-interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia is recognized to be heterogeneous. Acute exacerbation of IIPs is now well defined. A substantial percentage of patients with IIP are difficult to classify, often due to mixed patterns of lung injury. A classification based on observed disease behavior is proposed for patients who are difficult to classify or for entities with heterogeneity in clinical course. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histologic patterns, is introduced. The rapidly evolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determining diagnosis, and potentially prognosis and treatment.

CONCLUSIONS: This update is a supplement to the previous 2002 IIP classification document. It outlines advances in the past decade and potential areas for future investigation.

References

  1. Am J Surg Pathol. 2005 Jul;29(7):948-54 - PubMed
  2. Eur Respir J. 2009 Jan;33(1):68-76 - PubMed
  3. Eur Respir J. 2009 Jan;33(1):77-84 - PubMed
  4. Arch Pathol Lab Med. 2002 Sep;126(9):1064-70 - PubMed
  5. Am J Respir Crit Care Med. 2001 Nov 1;164(9):1722-7 - PubMed
  6. Am Rev Respir Dis. 1985 Jul;132(1):182-5 - PubMed
  7. Chest. 2004 Feb;125(2):522-6 - PubMed
  8. Mod Pathol. 2002 Nov;15(11):1148-53 - PubMed
  9. Am J Respir Crit Care Med. 2000 Sep;162(3 Pt 1):1109-14 - PubMed
  10. Respirology. 2006 Jan;11 Suppl:S51-4 - PubMed
  11. Am J Respir Crit Care Med. 2010 Dec 1;182(11):1419-25 - PubMed
  12. Tissue Antigens. 2008 Nov;72(5):483-6 - PubMed
  13. J Med Genet. 2008 Oct;45(10):654-6 - PubMed
  14. Am J Respir Crit Care Med. 2005 Oct 15;172(8):1026-31 - PubMed
  15. Am J Respir Crit Care Med. 2003 Aug 1;168(3):330-4 - PubMed
  16. Eur Radiol. 2012 Jan;22(1):83-92 - PubMed
  17. Thorax. 2002 Apr;57(4):338-42 - PubMed
  18. Am J Respir Crit Care Med. 2004 Sep 1;170(5):508-15 - PubMed
  19. Eur Respir J. 2002 Mar;19(3):439-46 - PubMed
  20. Respiration. 2005 Jan-Feb;72(1):39-45 - PubMed
  21. Radiology. 2006 Jan;238(1):321-9 - PubMed
  22. Radiology. 1993 Sep;188(3):817-20 - PubMed
  23. Mol Med. 2003 Jan-Feb;9(1-2):52-6 - PubMed
  24. Nihon Kokyuki Gakkai Zasshi. 2001 Aug;39(8):609-14 - PubMed
  25. Respirology. 2006 Mar;11(2):164-8 - PubMed
  26. N Engl J Med. 2011 Apr 21;364(16):1576-7 - PubMed
  27. Thorax. 2011 Jan;66(1):61-5 - PubMed
  28. AJR Am J Roentgenol. 1997 Jan;168(1):79-83 - PubMed
  29. J Comput Assist Tomogr. 2000 Jan-Feb;24(1):41-6 - PubMed
  30. Thorax. 2003 Jan;58(1):52-7 - PubMed
  31. Eur Respir J. 2011 Feb;37(2):356-63 - PubMed
  32. Int J Biol Markers. 2010 Oct-Dec;25(4):229-35 - PubMed
  33. Eur J Intern Med. 2008 Jun;19(4):227-35 - PubMed
  34. Histopathology. 2008 Dec;53(6):707-14 - PubMed
  35. Am J Surg Pathol. 2009 Dec;33(12):1765-70 - PubMed
  36. Eur Radiol. 2006 Nov;16(11):2463-70 - PubMed
  37. Radiology. 2001 Dec;221(3):600-5 - PubMed
  38. Eur Respir J. 2005 Oct;26(4):586-93 - PubMed
  39. Sarcoidosis Vasc Diffuse Lung Dis. 2009 Jul;26(2):155-61 - PubMed
  40. AIDS. 2007 Oct 1;21(15):2116-7 - PubMed
  41. J Thorac Imaging. 2007 Aug;22(3):221-9 - PubMed
  42. J Comput Assist Tomogr. 2003 Mar-Apr;27(2):260-5 - PubMed
  43. AJR Am J Roentgenol. 1990 May;154(5):983-7 - PubMed
  44. AJR Am J Roentgenol. 2003 May;180(5):1251-4 - PubMed
  45. Eur Respir J. 2006 Aug;28(2):364-9 - PubMed
  46. Eur Respir J. 2001 Jan;17(1):122-32 - PubMed
  47. Am J Surg Pathol. 2007 Feb;31(2):277-84 - PubMed
  48. Am J Surg Pathol. 2006 Feb;30(2):201-8 - PubMed
  49. Respiration. 2005 May-Jun;72(3):254-62 - PubMed
  50. Clin Chest Med. 2004 Sep;25(3):531-47, vi - PubMed
  51. Radiology. 2008 Apr;247(1):251-9 - PubMed
  52. Chest. 2004 Dec;126(6):2007-13 - PubMed
  53. Am J Respir Crit Care Med. 2004 Oct 15;170(8):904-10 - PubMed
  54. Semin Respir Crit Care Med. 2008 Dec;29(6):643-50 - PubMed
  55. Chest. 2009 Jun;135(6):1557-1563 - PubMed
  56. Chest. 2003 Oct;124(4):1199-205 - PubMed
  57. Chest. 1992 Jul;102(1 Suppl):8S-13S - PubMed
  58. Am J Respir Crit Care Med. 2002 Jan 15;165(2):148-51 - PubMed
  59. AJR Am J Roentgenol. 1999 Oct;173(4):949-53 - PubMed
  60. Eur Respir J. 2010 Jan;35(1):105-11 - PubMed
  61. J Comput Assist Tomogr. 2009 May-Jun;33(3):410-5 - PubMed
  62. Respir Med. 2005 Mar;99(3):265-7 - PubMed
  63. Am J Surg Pathol. 2000 Jan;24(1):19-33 - PubMed
  64. Radiology. 1999 Aug;212(2):567-72 - PubMed
  65. Eur Respir J. 2007 Jan;29(1):134-7 - PubMed
  66. Am J Respir Crit Care Med. 2009 Apr 15;179(8):717-23 - PubMed
  67. Am J Respir Crit Care Med. 2007 Oct 1;176(7):636-43 - PubMed
  68. Respir Med. 2009 Jun;103(6):846-53 - PubMed
  69. Am J Surg Pathol. 1994 Feb;18(2):136-47 - PubMed
  70. Thorax. 2008 Apr;63(4):366-73 - PubMed
  71. Radiology. 2007 Aug;244(2):591-8 - PubMed
  72. Chest. 2008 Dec;134(6):1265-1270 - PubMed
  73. Eur Respir J. 2012 Aug;40(2):377-85 - PubMed
  74. Chest. 2007 Mar;131(3):664-671 - PubMed
  75. Am J Respir Crit Care Med. 2008 Feb 15;177(4):433-9 - PubMed
  76. J Clin Pathol. 2006 Jan;59(1):28-39 - PubMed
  77. Chest. 2009 Jul;136(1):1-2 - PubMed
  78. Am J Respir Crit Care Med. 2008 Aug 15;178(4):372-8 - PubMed
  79. Am J Respir Crit Care Med. 2000 Aug;162(2 Pt 1):571-7 - PubMed
  80. J Comput Assist Tomogr. 2005 Nov-Dec;29(6):793-800 - PubMed
  81. Biochim Biophys Acta. 2009 Apr;1792(4):371-9 - PubMed
  82. PLoS One. 2010 Jan 29;5(1):e8959 - PubMed
  83. AJR Am J Roentgenol. 2010 Oct;195(4):916-22 - PubMed
  84. Thorax. 2004 Jun;59(6):506-11 - PubMed
  85. Thorax. 2004 Nov;59(11):977-80 - PubMed
  86. Eur Respir J. 2008 Mar;31(3):585-91 - PubMed
  87. Am J Respir Crit Care Med. 2007 May 15;175(10):1054-60 - PubMed
  88. Am J Respir Crit Care Med. 2003 Sep 1;168(5):531-7 - PubMed
  89. Eur Respir J. 2000 Feb;15(2):412-8 - PubMed
  90. Medicine (Baltimore). 2000 Nov;79(6):369-78 - PubMed
  91. Am J Respir Crit Care Med. 2002 Feb 1;165(3):378-81 - PubMed
  92. Hum Pathol. 2012 May;43(5):660-8 - PubMed
  93. Am J Respir Crit Care Med. 2008 Oct 1;178(7):729-37 - PubMed
  94. Ann Allergy Asthma Immunol. 2007 Nov;99(5):465-6 - PubMed
  95. Am J Respir Crit Care Med. 2012 Jan 1;185(1):67-76 - PubMed
  96. Radiology. 1999 May;211(2):555-60 - PubMed
  97. Pulm Pharmacol Ther. 2008;21(1):26-31 - PubMed
  98. Am J Respir Crit Care Med. 2002 Jun 1;165(11):1551-6 - PubMed
  99. Clin Chest Med. 2000 Mar;21(1):11-35, vii - PubMed
  100. Thorax. 2004 Jun;59(6):500-5 - PubMed
  101. AJR Am J Roentgenol. 1994 Mar;162(3):543-6 - PubMed
  102. AJR Am J Roentgenol. 2007 Feb;188(2):334-44 - PubMed
  103. Am J Respir Crit Care Med. 2002 Jan 15;165(2):277-304 - PubMed
  104. Radiology. 2008 Jan;246(1):288-97 - PubMed
  105. Respir Med. 2011 Dec;105(12):1902-7 - PubMed
  106. Thorax. 2000 Feb;55(2):143-6 - PubMed
  107. Mod Pathol. 2008 Jun;21(6):784-7 - PubMed
  108. J Assoc Physicians India. 2008 Apr;56:229-32 - PubMed
  109. Hum Genet. 2003 Nov;113(6):542-50 - PubMed
  110. Thorax. 2003 Feb;58(2):143-8 - PubMed
  111. Respir Med. 2010 Oct;104(10):1527-34 - PubMed
  112. Respir Med. 2009 Nov;103(11):1719-24 - PubMed
  113. Respir Investig. 2012 Sep;50(3):88-97 - PubMed
  114. Arch Intern Med. 1997 Jun 23;157(12):1323-9 - PubMed
  115. Am J Surg Pathol. 2004 Jan;28(1):62-8 - PubMed
  116. J Intern Med. 2006 Nov;260(5):429-34 - PubMed
  117. Radiology. 2000 Dec;217(3):701-5 - PubMed
  118. Radiology. 1999 Jan;210(1):29-35 - PubMed
  119. Am J Surg Pathol. 2002 May;26(5):647-53 - PubMed
  120. Respirology. 2008 May;13(3):408-12 - PubMed
  121. Arthritis Rheum. 2000 Jul;43(7):1633-40 - PubMed
  122. Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824 - PubMed
  123. Respiration. 2009;78(3):285-92 - PubMed
  124. Arthritis Rheum. 2007 May;56(5):1685-93 - PubMed
  125. Radiology. 2009 Apr;251(1):271-9 - PubMed
  126. Radiology. 2010 Mar;254(3):957-64 - PubMed
  127. Radiology. 1999 Jun;211(3):859-63 - PubMed
  128. Am J Respir Crit Care Med. 2007 Apr 1;175(7):705-11 - PubMed
  129. Am J Clin Pathol. 2003 May;119(5):709-14 - PubMed
  130. Sarcoidosis Vasc Diffuse Lung Dis. 2007 Mar;24(1):78-80 - PubMed
  131. Am J Respir Crit Care Med. 2007 Oct 1;176(7):691-7 - PubMed
  132. Eur Respir J. 2013 Sep;42(3):750-7 - PubMed
  133. Am J Respir Crit Care Med. 2005 Aug 15;172(4):488-93 - PubMed
  134. Chest. 2007 Jul;132(1):214-20 - PubMed
  135. Eur Respir J. 1998 Nov;12(5):1010-9 - PubMed
  136. Hum Genet. 2008 Dec;124(5):465-72 - PubMed
  137. Am J Med Sci. 2011 Jun;341(6):439-43 - PubMed
  138. Radiology. 2004 Sep;232(3):757-61 - PubMed
  139. Radiology. 2002 Oct;225(1):199-204 - PubMed
  140. PLoS Med. 2008 Apr 29;5(4):e93 - PubMed
  141. Am J Clin Pathol. 2009 Mar;131(3):405-15 - PubMed
  142. Am J Respir Crit Care Med. 1998 Oct;158(4):1286-93 - PubMed
  143. Sarcoidosis Vasc Diffuse Lung Dis. 2005 Oct;22(3):180-5 - PubMed
  144. Mod Pathol. 2011 Dec;24(12):1633-9 - PubMed
  145. N Engl J Med. 2001 Feb 22;344(8):573-9 - PubMed
  146. N Engl J Med. 2011 Apr 21;364(16):1503-12 - PubMed
  147. Chest. 2009 Jul;136(1):10-15 - PubMed
  148. Am J Respir Crit Care Med. 2007 Jan 1;175(1):45-54 - PubMed
  149. Lung. 2007 Jan-Feb;185(1):39-46 - PubMed
  150. Proc Natl Acad Sci U S A. 2008 Sep 2;105(35):13051-6 - PubMed
  151. Am J Physiol Lung Cell Mol Physiol. 2008 Jun;294(6):L1119-26 - PubMed
  152. Eur Respir J. 2012 Mar;39(3):661-8 - PubMed
  153. Mayo Clin Proc. 2007 Jul;82(7):812-6 - PubMed
  154. N Engl J Med. 1985 Jan 17;312(3):152-8 - PubMed
  155. Histopathology. 2004 Sep;45(3):275-82 - PubMed
  156. Radiology. 1995 Jun;195(3):645-8 - PubMed
  157. Ann Diagn Pathol. 2008 Jun;12(3):171-80 - PubMed
  158. Am J Respir Crit Care Med. 2006 Feb 1;173(3):310-7 - PubMed
  159. Am J Respir Crit Care Med. 2008 Jun 15;177(12):1338-47 - PubMed
  160. Hum Pathol. 2010 Mar;41(3):316-25 - PubMed
  161. Hum Genet. 2005 Nov;118(2):235-44 - PubMed
  162. AJR Am J Roentgenol. 1995 Oct;165(4):807-11 - PubMed
  163. Thorax. 2009 Feb;64(2):156-61 - PubMed
  164. Chest. 2009 Jul;136(1):23-30 - PubMed

MeSH terms

Publication Types